Characteristic Ultrastructural Findings in Metablic and Storage Diseases
スポンサーリンク
概要
- 論文の詳細を見る
Electron microscopic observations performed with immunoelectron microscopy and specific cytochemical stainings play an important role in the diagnosis of diseases affectingcarbohydrate, lipid, protein, and mineral metabolism. The characteristic ultrastructural changes in affected cels of metabolic diseases are summarized as follows: (1) many glycogen granules in glycogenosis type Ⅰ; (2) many glycogen granules in glycogenosis type Ⅲ; (3) glycogenosomes in glycogenosis type Ⅱ; (4) curvilinear tubular structures in Farber's disease; (5) mylin-like figures in Niemann-Pick's disease, and in splenic macrophages with idiopathic thrombocytopenic purpura; (6) tubular structures in hemolytic anemia resulting from overproduction of adenosine deaminase; (7) zebra bodies or membranous cytoplasmic bodies in Fabry's disease; (8) vacuoles containing ggranular and flocculent materials in Hurler's syndrome; (9) vacuoles in sialidosis; (10) vacuoles containing granular, flocculent and myelin-like figures in I-cell disease; (11) membrane-like structures in glycogenosis type Ⅳ; (12) membrabe-like structures showing a positive reaction with Thiery stains for demonstrating carbohydrate materials in Lafora's disease; (13) nonbranching fibrils, measuring 7-15 nm in width, in the extracellular space and rarely in a few cells from systemic amyloidosis and localized amyloidosis (using immunoelectron microscopy with antibodies and localized amyloidosis (using immunoelectron microscopy with antibodies to AA, Aλ, Aκ, β2-microglobulin, and β/A4, we have been able to identify amyloid fibrils in various kinds of amyloidosis); (14) vacuolar inclusions with limiting membrane varying in diameter from 200-300 μm to several μm in polyvinyl pyrrolidone thesaurosis; and (15) aurosomes in patients with Felty's syndrome who have received gold sodium thiomalate.
- Yamaguchi University Graduate School of Medicineの論文
著者
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GONDO Toshikazu
Department of Surgical Pathology, Yamaguchi University Hospital
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Takahashi Mutsuo
Department Of Surgical Pathology Yamaguchi University Hospital
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KAWANO Hiroo
First Department of Pathology, Yamaguchi University School of Medicine
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ISHIHARA Tokuhiro
First Department of Pathology, Yamaguchi University School of Medicine
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KOGA Mayumi
Department of Pediatrics, Yamaguchi University School of Medicine
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YOKOTA Tadaaki
Department of Pathology, Kokura Memorial Hospital
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UCHINO Fumiya
Yamato Town Hospital
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Yokota Tadaaki
Department Of Pathology Kokura Memorial Hospital
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Gondo T
山口大学 医学系研究科泌尿器科学
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Yamashita Y
Department Of Pathology Tokuyama Central Hospital
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Koga Mayumi
Department Of Pediatrics Yamaguchi University School Of Medicine
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Iwata Takako
Department Of Pathology School Of Medicine Yamaguchi University
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Kawano Hiroo
First Department Of Pathology Yamaguchi University School Of Medicine
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Hoshii Yoshinobu
First Department Of Pathology Yamaguchi University Graduate School Of Medicine
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Takahashi Masayo
Department Of Ophthalmology And Visual Sciences Graduate School Of Medicine Kyoto University
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Kawano Hisashi
Department Of Pediatrice Kyoto Prefectural University Of Medicine
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